Myeloma (Rustitsky-Kahler disease, Multiple myeloma, Plasmacytoma)

Myeloma is a pathological condition of the circulatory system, which is characterized by the presence of a neoplasm of lymphocytic plasma cells of malignant etiology. It causes the accumulation of abnormal “Ig” antibodies in the patient’s blood, which leads to disruptions of humoral immunity and destructive processes of bone tissue. Most often, the disease affects the bone marrow and bones, much less often – the lymph nodes, spleen, and intestines. Among all malignant neoplasms affecting lymphatic and hematopoietic tissues, the proportion of myeloma does not exceed 10%. It affects patients over forty years of age, much more often men.

The Department of Hematology at CELT offers diagnosis and treatment of multiple myeloma in Moscow. Being a multidisciplinary clinic, our clinic has a modern diagnostic and treatment facility, which allows for comprehensive, accurate diagnosis and treatment according to international standards. We use gentle techniques that give positive results, eliminating diseases and restoring the joy of life to our patients. You can find out the estimated cost of our services in the “Services and Prices” section on our website. Don’t forget to check the numbers with our information line operators.

Myeloma: causes

The reasons for the development of myeloma are still unclear. Experts put forward different theories. Quite often, patients are diagnosed with chromosomal mutations of various types, as well as congenital predisposition, which is inherited from parents to children. In recent years, there has been an increase in the number of patients, which is associated with exposure to various types of carcinogens and radioactive radiation. Quite often, malignant tumors are diagnosed in patients whose work involves contact with petroleum products, as well as in farmers and carpenters.

Uncontrolled growth of myeloma tissue in the bone marrow provokes the destruction of hematopoietic tissue and inhibition of myelo- and lymphopoiesis. As a result, the number of red and white blood cells and platelets in the blood decreases. Plasma cells cannot perform the immune function fully due to a serious decrease in the synthesis and abnormally rapid destruction of normal antibodies. Foci of bone tissue dissolution appear around the neoplasm: it becomes soft and brittle, and a large amount of calcium enters the blood.

Symptoms and signs of myeloma

As a rule, the disease is rarely detected in the early stages, since it usually does not bother its owner until the lesions become quite serious. Symptoms of myeloma may look like this:

Bone problems

:

• Pain in any area of ​​the body, but most often in the back, hips and skull.
• Osteoporosis is a decrease in bone density and disruption of its structure due to leaching of calcium from it - throughout the body or only in the place where the tumor is located.
• Fractures that occur due to minor trauma or light load.

Low blood counts

:

• Anemia: a reduced number of oxygen-carrying cells, which leads to weakness, inability to tolerate normal and habitual exercise, dizziness and shortness of breath - a feeling of lack of air.
• Thrombocytopenia: A lack of clot-forming platelets can cause severe bleeding from even minor abrasions, cuts or bruises.
• Leukopenia: a lack of white blood cells that protect us reduces resistance to various infections.

Hypercalcemia – high levels of calcium in the blood

due to its release from the bones:

• Intense thirst.
• Frequent urination.
• Dehydration.
• Problems with the kidneys, up to failure - organ failure.
• Severe constipation.
• Stomach ache.
• Loss of appetite.
• Weakness.
• Drowsiness.
• Confusion.
• If calcium levels rise critically, a person may even fall into a coma - an unconscious state.

Damage to the nervous system

. Weakened bones in the spine can become brittle, rupture, and put pressure on nerves, causing:

• sudden severe back pain;
• numbness, usually in the legs;
• muscle weakness, usually in the lower extremities.

Some proteins produced by myeloma cells are toxic - poisonous to nerves

. Their defeat can also lead to weakness, numbness and a tingling sensation.

In some patients, excess of these substances leads to “thickening” of the blood

–
increasing
its
viscosity
. This condition slows the flow of this vital fluid to the brain and can cause dizziness, disturbances in consciousness and the development of stroke symptoms. A stroke is an acute problem of blood circulation in the brain - weakness of muscles on one side of the body and slurred speech.

In addition, altered proteins damage the kidneys

. In the early stages of the disease, this does not cause any symptoms, but signs of their damage can be seen in blood or urine tests. When these organs begin to fail, they stop removing excess salt, fluid, and waste products from the body, resulting in weakness, itching, difficulty breathing, and swelling of the legs.

Myeloma patients often suffer from various infections

, most often from pneumonia - inflammation of the lungs, usually respond poorly to treatment and take a long time to recover.

Myeloma: symptoms

The initial stages of the development of the disease occur practically without symptoms. The patient feels well, and the tumor is discovered by chance during a laboratory blood test. Clinical manifestations of multiple myeloma, which affects the red bone marrow of the spine, ribs, pelvis and hips, begin with a feeling of pain in them. The latter occurs during movements or palpation. Spontaneous bone fractures are not uncommon. Particularly dangerous are compression fractures of the spine, which provoke:

• spinal cord compression;
• impaired sensitivity of the limbs;
• disruptions in the functioning of the intestines and organs of the urinary system.

Symptoms of myeloma amyloidosis include manifestations of damage to various internal organs, including the heart, gastrointestinal tract, as well as the eyes, tongue, joints, and skin:

• heart rhythm disturbances in the form of tachycardia;
• heart failure;
• increase in tongue tissue;
• difficult and painful digestion;
• degenerative processes of the cornea;
• deformation processes of joints;
• systemic lesions of peripheral nerves.

Severe myeloma is characterized by:

• increased urine production;
• nausea and vomiting;
• dehydration of the body;
• feeling of weakness in the muscles;
• fatigue and drowsiness;
• in some cases - coma.

Due to the fact that the blood becomes more viscous, its microcirculation is disrupted. The patient becomes susceptible to the effects of pathogenic bacterial agents, so even at an early stage of myeloma, he can develop pneumonia or pyelonephritis, the course of which will be quite severe. Complications from infectious diseases are one of the main causes of death in myeloma.

Diagnostics

To diagnose the disease you need:

• taking an anamnesis, including identifying factors that indicate a predisposition to developing the disease (for example, age, heredity, previous diseases);
• blood and urine tests;
• bone marrow puncture;
• trepanobiopsy;
• Ultrasound, x-ray of bones;
• MRI;
• spiral computed tomography;
• Lumbar puncture.

From the proposed list of methods, the doctor selects the most appropriate research in a particular case. When clarifying the diagnosis, the use of techniques involving the administration of contrast agents is prohibited, since in this case it is very dangerous for the kidneys.

How to treat multiple myeloma?

In order to get the best results and improve the patient’s quality of life, CELT hematologists develop treatment tactics immediately after diagnosis. They are guided by data from diagnostic studies and individual patient indications. If myeloma develops slowly, watchful waiting with close monitoring until symptoms increase is indicated.

After completing the course of treatment, the disease recurs over the next twelve months. Each subsequent remission lasts less than the previous one and is achieved with great difficulty. In order to prolong it, the patient is prescribed maintenance therapy using interferon “A” drugs. Complete recovery is possible only in 10% of cases.

The Department of Hematology at CELT welcomes candidates, doctors and professors of medical sciences with over twenty-five years of practical and scientific experience. You can make an appointment with them online or by contacting our information line operators. Being a multidisciplinary clinic, our clinic offers the services of highly qualified otolaryngologists. Our patients have access to septoplasty, thanks to which they can improve their quality of life.

At CELT you can consult a hematologist.

• Initial consultation – 3,500
• Repeated consultation – 2,300

Make an appointment
By making an appointment with a hematologist, you can get a comprehensive consultation. The doctor is competent to treat various blood diseases, most of which can be identified in the early stages and prescribe timely treatment to cope with the disease quickly and easily.

Classification

Plasma cell tumors include:

• Multiple myeloma.
• Plasma cell myeloma and its variants (asymptomatic, non-secreting plasma cell leukemia).
• Plasmacytoma.

According to the activity of the disease, there are:

• “Smoldering” (asymptomatic). This form does not manifest symptoms characteristic of the disease (there are no foci of bone destruction and damage to internal organs). The M component is more than 30 g/l or the number of plasma cells in the bone marrow is more than 10%. The risk of progression to symptomatic disease is high.
• Active myeloma. There is active tumor growth, osteodestruction , increased blood viscosity, and infectious complications appear.

According to the clinical and anatomical classification, multiple myeloma comes in several forms:

• Diffuse.
• Multifocal.
• Diffuse-focal form.
• Mainly visceral.

Multiple myeloma (ICD-10 code C90.0) has stages that differ in the degree of production of pathological protein:

• Stage I. Low production of M-protein (IgG less than 30 g/l), tumor weight up to 600 g/m2, protein B-D less than 4 g/s, normal bone structure or there is one lesion.
• Stage II. The tumor weight is 600-1200 g/m2 and the rate of M-complex production is average.
• Stage III. Low hemoglobin level, elevated calcium, multiple bone lesions, IgG more than 70 g/l, and protein B-D above 12 g/day.

The blood disease myeloma can be quiet and symptomatic with bone damage, bone marrow suppression and kidney failure. Symptomatic myeloma blood disease is characterized by high production of paraprotein and it corresponds to the “detailed” picture of the disease and necessarily requires treatment. Impaired renal function is always present and, depending on the level of creatinine, all patients are divided into three groups to determine the prognosis.

Main criteria of the disease:

• Detection of more than 10% plasma cells in the bone marrow or the development of a plasma cell tumor.
• The presence of monoclonal immunoglobulin , which is produced by malignant cells.
• It is determined by electrophoresis of blood proteins. In practice, they focus on a value of 30 g/l IgG. it is necessary to constantly monitor the level of M-protein, since its increase is a progression factor.
• Often, patients monoclonal immunoglobulin , but there is a decrease in the level of gammaglobulin in the blood and when examining urine, the release of light k-chains is noted.

When organs and tissues are damaged, the following develop in parallel:

• Calcium levels increase.
• Renal function is impaired (the main criterion is increased creatinine).
• Anemia develops .
• Bone damage (compression of vertebral bodies, lysis, fractures).

Damage in myeloma is diffuse and focal. In the diffuse form, only the bone marrow is affected, and this form is detected in 24% of cases. The diffuse-focal form of multiple myeloma is a mixed variant and combines the presence of tumor growth in the body and damage to the bone marrow. This option is most common.

Nonsecretory myeloma is said to occur when plasma cells are detected in the bone marrow and there are signs of organ damage. But with this form there is no monoclonal protein in the blood and urine, so kidney failure rarely develops.

Asymptomatic or smoldering form. It is characterized by the following criteria: M protein more than 30 g/l, 500 mg of paraprotein in the urine or more than 10% of plasma cells in the bone marrow. There is no damage to organs and bones, and there is no amyloidosis . In this case, treatment is not carried out, but patients are observed once a quarter (paraprotein in the blood and urine is determined). Typically, the interval before disease progression is 4 years.

Plasmacytomas are plasma cell tumors. Depending on the location, they can be bone (bone plasmacytoma) or extramedullary (soft tissue is affected). Solitary plasmacytoma is represented by a single focus in the bone or tissue (it happens that the production of M protein is low). The diagnosis of bone plasmacytoma is made if the tumor is located in the bone and there are no other systemic signs. The disease is more common in young people. Solitary lesions are located in the vertebrae ( vertebral plasmacytoma ) and tubular bones. Patients are concerned about pain in damaged bones and pathological fractures. For solitary plasmacytoma, radiotherapy is used. In 50%, transformation to multiple myeloma is noted within 3 years.

Cutaneous plasmacytoma or extramedullary myeloma of the skin has a favorable course. Skin lesions in extramedullary myeloma occur in 4% of patients. Unlike multiple myeloma, cutaneous plasmacytoma occurs without damage to the bone marrow and organs. If there is no hypercalcemia and no bone marrow involvement, their life expectancy can reach 10 years. But there are observations when, with cutaneous myeloma, myelomatosis develops after a few years . There are also opposite cases - myeloma metastasizes to the skin from the bone marrow. This fact is considered as an unfavorable sign, and the life expectancy of patients can be 1.5-12 months.

Causes and risk groups

The exact causes leading to the occurrence of this disease have not been identified. Presumable etiological factors are:

1. Ionizing radiation.
2. Prolonged contact with toxic substances.
3. Long-term antigenic stimulation.
4. Infection with herpes virus type 8.

The effect of a hereditary factor for myeloma is doubtful, but sometimes it occurs in members of the same family.

Risk factors for multiple myeloma include:

1. Age over 40 years (average age of patients is 65 years).
2. Male gender.

Popular questions about myeloma

Is it possible to recover from multiple myeloma?

Currently, bone myeloma is considered an incurable but fairly well-controlled disease with long-term remission. A bone marrow transplant provides the best chance for a complete cure.

How does myeloma manifest?

Multiple bone myeloma is manifested by pathological destruction of bones, frequent fractures, deterioration of blood and urine parameters (increased protein content), as well as kidney function. On x-ray it looks like multiple foci of destruction, which often lead to fractures. The patient feels weak and paralysis of the limbs occurs.

How long can a person live with myeloma?

On average, after starting treatment, patients live 2–2.5 years. Maximum – up to 10 years.

Treatment

The selection of drugs for myeloma and the treatment method are selected depending on the stage of the disease. Active therapy is prescribed when the disease develops intensively and the tumors increase in size. Prescribed:

• bone marrow transplant. This is the most effective way to cure this disease;
• chemotherapy - taking drugs that quickly rid the body of abnormal cells. The result of this method is assessed no earlier than after 3 months;
• local radiation therapy. In this case, certain parts of the patient's body are irradiated. The method is effective when tumor nodes are limited. It should be understood that in the irradiated area the probability of disease recurrence increases 5 times;
• restoration of normal mineral and protein metabolism. For this purpose, calcium, vitamin D, and steroids are used;
• correction of renal failure, restoration of normal renal function;
• surgical techniques (according to indications);
• extracorporeal techniques, the essence of which is blood purification with special devices (hemodialysis, plasmapheresis, hemosorption);
• transfusion of red blood cells (necessary for anemia, including those accompanied by a coma);
• physiotherapy;
• taking immunomodulatory drugs.